1,001 Nights

For eight years, every night is the same. The same routine of countless details, connected in precise order, each mundane, each crucial.

The routine makes the eight years seem longer, until afterward, when because of the routine the years seem to have evaporated.

Wake up to a steady, motorized noise. Something wrong with the water heater. Nnngah. Pause. Nnngah. Nnngah.

But it's not the water heater. It's my boy, Walker, grunting as he punches himself in the head, again and again.

He has done this since before he was 2. He's 11 now. He was born with an impossibly rare and random genetic mutation  cardio-facio-cutaneous syndrome, a technical name for a mash of symptoms. He is globally delayed and can't speak, so I never know what's wrong. No one does.

There are possibly 300 people with CFC around the world. Doctors call it an orphan syndrome.

I count the grunts on the way to his room: one a second. To get him to stop hitting himself, I have to get him back to sleep, which means taking him downstairs and making him a bottle and bringing him back into bed with me.

That sounds simple enough, doesn't it? But with Walker, everything is complicated. Because of his syndrome, he can't eat solid food by mouth, or swallow easily. Because he can't eat, he's fed formula through the night via a feeding system. The formula runs along a line from a feedbag and a pump on a metal IV stand, through a hole in Walker's sleeper and into a clever-looking permanent valve, sometimes known as a G-tube or a mickey, in his belly. To take him out of bed and down to the kitchen to make the bottle that will ease him back to sleep, I have to disconnect the line from the mickey.

To do that, I first have to turn off the pump (in the dark, so he doesn't wake up completely) and close the feed line. If I don't clamp the line, the sticky formula pours out onto the bed or the floor (there are patches of carpet that feel like the Gobi Desert under my feet, from all the times I have forgotten). To crimp the tube, I thumb a tiny red plastic roller down a slide. (It's my favourite part of the routine  one thing, at least, is easy.) I unzip his one-piece sleeper (Walker's small, and grows so slowly he wears the same sleepers for a year and a half at a time), reach inside to unlock the line from the mickey, pull the line out through the hole in his sleeper and hang it on the IV rack that holds the pump and feedbag.

Then I reach in and lift all 45 pounds of Walker from the depths of the crib. He still sleeps in a crib. It's the only way we can keep him in bed at night. He can do a lot of damage on his own.

This isn't a list of complaints. There's no point to complaining. As the mother of another CFC child once told me, "You do what you have to do." If anything, that's the easy part.

The hard part is trying to answer the questions Walker raises in my mind every time I pick him up. What is the value of a life like his  a life lived in the twilight, and often in pain? What is the cost of his life to those around him? "We spend a million dollars to save them," a doctor said to me recently. We were sitting in her office, and she was crying. "But then when they're discharged, we ignore them."

Sometimes, watching him, it's like looking at the moon: You see the face of the man in the moon, but you know there's actually no man there. But if Walker is so insubstantial, why does he feel so important? What is he trying to show me?

To answer that question, I decided to look again at the life he had lived, and the way we had helped him live it  first at home, later in a special community for children like him. I climbed into a car and drove across the continent to meet other children with his syndrome.

All I really want to know is what goes on inside his off-shaped head, in his jumped-up heart. But every time I ask, he somehow persuades me to look into my own.

Before I can slip downstairs with Walker for a bottle, the bloom of his diaper pillows up around me. He's not toilet-trained. Without a new diaper, he won't fall back asleep or stop smacking his head and ears. Detour to the routine of the diaper.

I spin 180 degrees to the battered changing table, wondering, as I do every time, how this will work when he's 20 and I'm 60. The trick is to pin his arms to keep him from whacking himself. But how do you change a 45-pound boy's brimming diaper while immobilizing both his hands so he doesn't bang his head or (even worse) reach down to scratch his tiny, plum-like but suddenly liberated backside, thereby smearing excrement everywhere? While at the same time immobilizing his feet, because ditto? You can't let your attention wander for a second. All this is done in the dark as well.

But I have my routine. I hold his left hand with my left hand, and tuck his right hand out of commission under my left armpit. I've done it so many times, it's like walking. I keep his heels out of the danger zone by stopping his knees from bending with my right elbow, and do all the actual nasty business with my right hand. My wife, Johanna, can't manage this alone any longer and sometimes calls me to help her. I am never charming when she does.

And the change itself  a task to be approached with all the delicacy of a munitions expert defusing an atomic device in a Bond movie. The unfolding and positioning of a new nappy; the immense, surging relief of finally fastening it up  we made it! The world is safe again! The reinsertion of his legs into the sleeper.

Now we're ready to head downstairs to make the bottle.

Three flights, taking it in the knees, looking out the windows as we go. He's stirring, so I describe the night to him. Tonight, there's no moon and it's damp for November.

In the kitchen, I perform the now-ancient bottle ritual. The weightless plastic bottle (the third shape we tried before we found one that worked, big enough for his not-so-fine motor skills yet light enough to hold), the economy-sized vat of Enfamil (whose bulk alone is discouraging, it implies so much), the tricky one-handed titrating of tiny tablespoonfuls of Pablum and oatmeal (he aspirates thinner fluids). The nightly pang about the fine film of Pablum dust everywhere: Will we ever again have anything like an ordered life? The ebb for having such thoughts in the first place. The rummage in the ever-present blue and white dish drainer (we're always washing something, a pipette or a syringe or a bottle or a medicine measuring cup) for a nipple (but the right nipple, one whose hole I have enlarged into an X, to let the thickened liquid out) and a cap. Pull the nipple into the cap, the satisfying pop as it slips into place. The gonad-shrinking microwave.

Back up three flights. He's still trying to smash his head. Why does he do it? Because he wants to talk, but can't? Because  this is my latest theory  he can't do what he can see other people doing? I'm sure he's aware of his own difference.

Cart him into his older sister Hayley's room. Hayley, meanwhile, is downstairs with her mother, so they can get some sleep. We take turns like this, reduced by the boy to bedroom Bedouins. Neither one of us has slept two full nights in a row in eight years. We both work during the day. After the first few months, I stopped noticing much difference.

Lay him down on the bed. Oh, fuck me dead  forgot the pump! Build a wall of pillows around him so he doesn't escape while I nip back. Remember four (or is it six?) cc's of chloral hydrate, prescribed for sleep and to calm his self-mutilation. (I tried a dose once: the kick of a double martini. William S. Burroughs was thrown out of school for experimenting with it.) Reprogram the pump, his night pulse.

At last: Sink into bed beside him, pull the wriggling boy close. He begins to hit his head again, so I hold down his small right hand with my large right one. This brings his left hand up to his other ear  "he's a genius for finding ways to hurt himself," his teacher told me the other day. I grab his left in my left, which I have threaded behind his head. He kicks himself in the crotch with his right heel, so hard it makes me wince: I run my big leg over his little leg, and lay my right hand (holding his right hand) on his left thigh, to keep it still. He's stronger than he looks. Under his birdy limbs, he's granite to his core. He'll mash his ears to a pulp if no one stops him.

There is a chance none of this will work. Every once in a while, the chloral hydrate rebounds and transforms him into a giggling drunk. When he has a cold (eight, 10 times a year), he wakes on the hour. Sometimes we perform the entire routine again an hour later. Sometimes he cries for hours. There are nights when nothing works, and nights when he is up and at it, laughing and playing and crawling all over me. I don't mind those nights, tired as I am: His sight is poor, but in the dark we're equal, and I know this makes him happy. In the night, there can be stretches when he is no different from any normal lively boy. It makes me almost cry to tell you that.

Tonight is a lucky night: I can feel him slip off after 10 minutes. He stops grunting, strokes his bottle, turns his back and jams his bony little ass into my hip, a sure sign. He falls asleep.

I hurry after him. For all this nightly nightmare  eight years of desperate worry and illness and chronic sleep deprivation, the havoc he has caused in our lives, threatening our marriage and our finances and our sanity  I long for the moment when he lets his crazy formless body fall asleep against me. For a short while, I feel like a regular little boy's father. Sometimes I think this is his gift to me  but parcelled out, to show me how rare and valuable the gift is. Walker, my teacher, my sweet, sweet, lost and broken boy.

Single-cell disorders

The chance of a disorder caused by a single gene is smaller, but still not as small as one might like. Some examples, and their incidence:

Sickle cell anemia:

First molecular disease to be identified, causing fatigue, delayed development, pulmonary hypertension, stroke and heart failure; 1 in 400 to 1 in 625 persons at birth among African-Americans

Familial hypercholesterolemia:

Coronary heart disease by middle age; in men who have inherited the trait from both parents, a 50-per-cent risk of heart attack by the age of 50; 1 in 500 males

Cystic fibrosis:

Chronic pulmonary disease, death by late 30s, most common autosomal recessive disease in Caucasian children; 1 in 2,000

Neurofibromatosis type 1:

The most common single-gene disorder, causing changes in skin colouring, tumours along nerves, loss of vision, leukemia, scoliosis, ADHD; 1 in 3,000 to 1 in 4,000 worldwide (though very rare in Asians)

Neurodegenerative disease with progressive dementia, with onset typically in the fifth decade; between 4 and 8 in 100,000

Alstrom syndrome:

Loss of vision and hearing, enlargement of heart, obesity, type 2 diabetes; first appears as sensitivity to light in infants; 425 known cases, worldwide

CFC syndrome, Costello Syndrome:

Facial deformity, heart defects, mild to severe mental retardation; risk of cancer in Costello syndrome, but not in CFC; 300 people worldwide, or 1 in 1.24 million (fewer than 10 births a year, worldwide)

Proteus syndrome:

Excess growth of tissue in body, asymmetrical growth, benign tumours; John Merrick, the famous “elephant man,” probably had Proteus syndrome; 100 to 200 people worldwide

Sources: Genetics in Medicine, W. B. Saunders Co, Philadelphia, 1991; National Down Syndrome Society; U.S. National Library of Medicine; Costello Syndrome Support group. With files from Marjan Farahbaksh, Globe and Mail Editorial Research Library.